【摘要】 目的 探討新診斷的多發性肌炎（PM）、皮肌炎（DM）和無肌病性皮肌炎（ADM）肺部病變發生率、臨床特點及相關因素。 方法 回顧性分析2008年1月—2010年7月新確診的206例PM、DM、ADM臨床表現、肺部影像學、肺功能、超聲心動圖和實驗室指標。 結果 206例患者中合并肺部病變156例，以肺間質病變（ILD）最多見，占患者總數的51.46%。在性別、病程、是否吸煙方面合并ILD與無ILD患者相比差異無統計學意義，合并ILD患者年齡大于無ILD組。合并ILD患者呼吸困難、發熱、雷諾現象、關節炎或關節痛概率增加。合并ILD組白蛋白低于無ILD組，血沉和免疫球蛋白IgM高于無ILD組。急性ILD組中女性患者及出現雷諾現象的概率高于慢性組。206例患者中死亡13例，其中周圍型肺癌1例，特發性血小板減少并顱內出血1例，嚴重肺部感染11例；死亡患者中10例伴肺間質纖維化。合并急性ILD患者死亡率較慢性組高2倍。 結論 PM、DM、ADM患者肺部病變發生率高，以ILD多見，發熱、年齡大、白蛋白降低、血沉升高、雷諾現象及關節炎或關節痛都是合并ILD的相關因素。合并急性ILD患者預后差，死亡者常合并肺部感染。【Abstract】 Objective To explore the prevalence, clinical features, and predictive factors of pulmonary involvement in newly diagnosed polymyositis (PM), dermatomyositis (DM) and amyopathic dermatomyositis (ADM), in order to carry out early diagnosis and treatment, and improve the prognosis. Methods The clinical manifestations, chest imaging, pulmonary function test, ultrasonic cardiography and laboratory results of 206 inpatients with PM, DM and ADM in West China Hospital of Sichuan University from January 2008 to July 2010 were reviewed retrospectively. Results One hundred and fifty-six out of 206 patients developed PM/DM/ADM associated lung diseases, including 106 cases (51.46%) of interstitial lung disease (ILD). There was no significant difference in gender, disease duration, and smoking or not between the ILD and non-ILD group, but patients in the ILD group were significantly older than non-ILD group. The results also showed that patients with ILD were much more likely to have symptoms of breathing difficulties, fever, Raynaud phenomenon and arthritis/arthralgia. The patients with ILD had lower level of albumin but higher levels of ESR and IgM; In the group of acute ILD, female patients and the ratio of Raynaud phenomenon were higher than those in the chronicity group. Of the 206 patients, 13 patients died, including 1 death of peripheral lung cancer, 1 of essential thrombocytopenia and intracranial hemorrhages, and 11 of severe lung infection, and 10 in these patients developed ILD. Mortality in patients with acute ILD was 2 times higher than the chronicity group. Conclusion The prevalence of lung diseases is high in patients with PM, DM and ADM. ILD is the main pulmonary involvement, and fever, older age at onset, hypoalbuminemia, higher values of blood sedimentation, Raynaud phenomenon and arthritis or arthralgia were the predictive factors for developing ILD. Patients with acute ILD have poor prognosis. Death cases often have pulmonary infections.
